June 25, 2007

Klippel-Feil Anomaly

This condition is a concern to neurologists because the anomaly, congenital fusion of cervical vertebrae, can sometimes have neurologic consequences. The vertebrae aren't actually fused; they fail to segment normally between the third and eighth weeks of development. On examination these patients have short necks, low hairlines, and limitation of neck movement. A recent example had one shoulder lower than the other. The picture in Bradley and Daroff (from where all information is borrowed and adapted) seems to demonstrate the same. Imaging reveals the fused vertebrae. Compression of nerve roots, cervical spinal cord, or vertebral or spinal artery can occur. Interestingly, suspected incomplete decussation of corticospinal fibers can cause mirror movements in children. There are many associated abnormalities, including bifurcated spinal cord. Many patients do just fine. The patient I saw had two discrete transient events characterized as right hand, lower arm, neck and face tingling. Neuroanatomically these events were difficult to ascribe to his K-F anomaly. DWI was negative. There was no history suggestive of an epileptic or migrainous phenomenon.